BackgroundInterstitial lung disease (ILD) in primary Sjögren’s syndrome (pSS) has been reported to be present in 10-15% of patients, but pSS-ILD behavior over time is not well characterized.ObjectivesAssess the pattern of ILD in pSS, its disease behavior and factors associated with disease progression in a well-characterized pSS-ILD cohort.MethodsAll pSS patients from the Oslo University Hospital (OUH) were included if ILD was diagnosed on HRCT. Clinical characteristics, lung function tests including forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) and ILD pattern on HRCT assessed by a radiologist were evaluated. We determined ILD progression, defined as absolute FVC decline >5% or absolute DLCO decline >10% over 12 +/-6 months and increasing extent of ILD on HRCT over the observation period. Factors associated with disease progression were chosen based on expert opinion. Descriptive analyses were conductedResultsOf 702 pSS patients followed at OUH, we identified 60 pSS patients with ILD with 33 (55%) having follow-up at 12 months (Table 1). Patients with pSS-ILD were characterized by high number of males (18%) and by frequent other extra-pulmonary organ involvement (48%) (Table 1). Mean time from pSS diagnosis to ILD diagnosis was 7.4 years. In 67% ILD was diagnosed after pSS, in 13% simultaneously, in 11% before pSS diagnosis and in 9% unknown. In total, 28 (47%) were diagnosed with lymphocytic interstitial pneumonia (LIP) and 32 (53%) with reticular pattern on HRCT. Over mean follow-up of 10.9 months (SD 4.2), 7/33 (21%) showed a FVC >5% decline, 9/32 (28%) a DLCO >10% decline and 12 (36%) had at least one of these defined lung function declines on standard of care treatment. Treatment was registered as ever used and by any indication. Over an observation period of 15.4 (SD 10.6) years, 27/47 (45%) showed any ILD progression on HRCT. HRCT pattern was not associated with risk of >10% DLCO decline or ILD progression on HCRT. >5% FVC decline occurred more frequently in patients with reticular pattern compared to LIP (6/17 (35%) vs 1/16 (6%), p=0.041). Factors significantly associated with ILD progression on lung function included higher baseline FVC (99% (SD16.4) vs 87% (SD14.9), p=0.032), higher DLCO (81% (SD13.1) vs 67% (SD17.4), p=0.020), increased CRP (2/10 (20%) vs 0/16 (0%), p=0.045) and presence of polyneuropathy (2/9 (22%) vs 1/17 (6%), p=0.045).Table 1.Clinical characteristics, demographics and outcome of pSS with ILDpSS-ILD(n=60)Age at pSS diagnosis, y (SD)50 (21.9)Time from pSS to ILD diagnosis, y (SD)7.4 (8.9)Male sex, n (%)11 (18)Anti-SSA AB, n/50 (%)46 (92)Increased CRP, n/47 (%)7 (15)Low complements, n/49 (%)5 (10)Extra-pulmonary involvement, n/46 (%)22 (48)Deceased, n (%)10 (17)Pulmonary involvementFVC% predicted (SD)91 (18.7)FVC decline>5%, n/33 (%)7 (21)DLCO% predicted (SD)70 (20.7)DLCO decline >10%, n/32 (%)9 (32)ILD progression on HRCT, n/47 (%)27 (45)Treatment during follow upRituximab, n (%)11 (18)Any other immunosuppressive, n (%)20 (33)Hydroxychloroquine, n (%)16 (27)Nintedanib, n (%)1 (2)Lung transplant, n (%)1 (2)ConclusionA substantial number of patients with pSS-ILD progressed during the time of observation. This highlights the importance of close monitoring and active consideration of treatment options in pSS-ILD. Recommendations for disease management including screening, diagnosis, disease monitoring and treatment for pulmonary involvement in pSS are lacking to date, but are highly needed.Disclosure of InterestsAnna-Maria Hoffmann-Vold Speakers bureau: Actelion, Boehringer Ingelheim, Jansen, Lilly, Medscape, Merck Sharp & Dohme, Roche, Consultant of: Actelion, ARXX, Bayer, Boehringer Ingelheim, Jansen, Lilly, Medscape, Merck Sharp & Dohme, Roche, Grant/research support from: Boehringer Ingelheim, Håvard Fretheim Consultant of: Bayer, Grant/research support from: Jansen, Phuong Phuong Diep Speakers bureau: Boehringer Ingelheim, Karoline Lerang: None declared, Helena Andersson: None declared, Øyvind Midtvedt: None declared, Torhild Garen: None declared, Mike Durheim Speakers bureau: Boehringer Ingelheim, Consultant of: Boehringer Ingelheim and Roche, Grant/research support from: Boehringer Ingelheim and Roche, Trond M Aaløkken Speakers bureau: Boehringer Ingelheim, Øyvind Palm: None declared, Øyvind Molberg: None declared
BackgroundPopulation-based studies on Systemic Lupus Erythematosus (SLE) patients with a verified diagnosis is considered the gold standard to find true outcomes in SLE, but few population-based SLE cohorts have follow-up over 15 years [1]. Norway is among the few countries worldwide where social and structural factors facilitate the gathering of complete population-based cohorts in rare disease like SLE due to its healthcare organization.ObjectivesTo examine long-term outcome of SLE in a population-based setting and determine if immediate cause of death differs between SLE patients and the general population.MethodsThe study included all SLE patients who were resident in the Southeast region of Norway during 1999 - 2017 and met the 1997 American College of Rheumatology classification criteria for SLE. All SLE diagnosis was confirmed by chart review. SLE patients and 15 controls for each case (matched by age, gender and ethnicity) were linked to the Norwegian Cause of Death Registry. We examined survival by means of Kaplan-Meyer estimates and used log rank test to test for differences. To estimate risk of death, we performed calculations of standard mortality rate (SMR) by dividing the number of deaths on the number of years observed. The excepted number of deaths referred to the number of deaths for the matched control group. All SLE cases were included in SMR. The 95 % confidence interval (CI) of SMR was calculated with Mid-P exact test. We defined immediate cause of death as the final event directly leading to death. An International Classification of Diseases 10th revision code of I00-99 or R96 classified as death from cardiovascular disease (CVD) (except pulmonary embolism and cerebral bleeding) and of infections A00-B99, J10-18, N39, M86 or U07.ResultsWe identified 1298 SLE patients in the region, of whom 673 was incident cases; all captures within one year from diagnosis. Of the incident cases, 76 (11%) died during 8434 years of follow-up (Table 1). The five-, ten-, 15- and 20-year survival for incident SLE patients (controls) was respectively 98 (98), 94 (96), 87 (94) and 82 (88) % and differed significantly first after ten years of disease duration compared to controls. Figure 1 shows 20-year survival for incident SLE patients and matched controls; stratified by gender. SMR for all SLE cases was 2.3 (95 % CI 1.5. - 4.0); female SLE 2.5 (95 % CI 1.6 – 3.9) and male SLE 1.9 (95 % CI 1.3 – 2.2). The most common immediate cause of death in SLE patients was CVD; whereof myocardial infarction (21 %) was most frequent. SLE patients died more often of CVD than controls (29 % vs. 21 %, p = 0.01) and had a tendency to more infections (23 % vs. 18 %, p = 0.07), whereof pneumonia (58 %) was most frequent.Table 1.Patient demographics, follow-up time and number of deaths in the total Systemic Lupus Erythematosus (SLE) cohort and in incident SLE patients.Total SLE cohortIncident SLEFemaleMalen = 1298n = 577n = 96Of European descent, n (%)1140 (88)472 (82)86 (90)Juvenile onseta, n (%)93 (7)31 (5)6 (6)LNb, n (%)470 (36)177 (30)49 (51)Cumulative ACR criteriac, µ (SD)5.4 (1.2)5.3 (1.2)5.1(1.1)Follow-up years, total1925261601217Deaths, n (%)282 (23)54 (9)22 (23)Age at diagnosis, years µ (SD)35.5 (15.7)37.4 (15.6)44 (17.9)Disease duration at death, years µ (SD)20.4 (12.5)9.6 (5.8)10.6 (10.5)µ: mean, n: number, SD: standard deviationa Diagnosed before age of 16 b Lupus Nephritis defined by 1999 American College of Rheumatology classification criteria for Systemic Lupus Erythematosus c1997 American Collee of Rheumatology classification criteria for Systemic Lupus ErythematosusConclusionMortality in SLE is substantially increased. Differences in survival compared to the general population only appear after ten years of disease duration. CVD was the most common immediate cause of death and more frequent in SLE patients.References[1]Reppe Moe, S., Haukeland, H., Molberg, Ø., & Lerang, K. (2021). Long-Term Outcome in Systemic Lupus Erythematosus; Knowledge from Population-Based Cohorts. J Clin Med, 10(19). doi:10.3390/jcm10194306Disclosure of InterestsNone declared
BackgroundBechet’s disease (BD) is a multisystemic, inflammatory disease, characterized by variety of clinical manifestations, including mucocutaneous, ocular, vascular, neurological (CNS) and gastrointestinal (GI) involvements. The disease can be seen at any age and occurs across gender, ethnic groups, and geographical areas. However, there are only a few studies from northern Europe.ObjectivesTo study the demographics and clinical feature of patients with Bechet’s disease (BD) in Oslo Norway.MethodsIn this retrospective study we reviewed hospital records from Oslo University Hospital (OUS), a referral hospital for systemic inflammatory vasculitis in Norway. All patients living in Oslo with ICD-10 code M35.2 were identified, and their BD-related data recorded. Those fulfilling either the International Study Group criteria (ISG) or International Criteria for Bechet’s disease (ICBD) were included. Patients were defined as non-Norwegian when none of the parents was of Norwegian ancestry. The differences between groups were compared by Chi-square test using the Statistical Package for the Social Sciences (SPSS).ResultsWe identified a total of 58 patients fulfilling the inclusion criteria (Table 1). Among them, 30 (52%) were females and 25 (43%) of Norwegian ancestry. Mean age at onset was 26 years, and at diagnosis 32 years.Table 1.Demographics of Behcet`s patients in Oslo NorwayCounts (%)Norwegian ancestryNon-Norwegian ancestryAll (%)All (%)25 (43)33 (57)58 (100)Male (%)8 (29)20 (71)28 (48)Female (%)17 (57)13 (43)30 (52)ISG Criteria (%)20 (47)23 (53)43 (100)ICBD Criteria (%)25 (43)33 (57)58 (100)Age at diagnosis mean (SD) years33 (11.85)32 (9.36)32 (10.41)Age at onset mean (SD) years24 (9.34)27 (9.52)26 (9.42)The most prevalent clinical manifestations were oral ulcers (n=58, 100%), genital ulcers (n=49, 84%) and skin lesions (n=40, 69%). Ophthalmic complications (n=21, 36%), arthritis (n=20, 34%), vascular involvement (n=7, 12%), CNS (n=3, 5%) and gastrointestinal (GI) complications (n= 4, 7%) were also present. There was no significant difference of clinical features between patients with Norwegian ancestry and those with non-Norwegian ancestry (data not shown).Figure 1.ConclusionBD was most prevalent among patients of non-Norwegian ancestry. We found a wide spectrum of clinical manifestations occurring during the disease course. Mucocutaneous lesions were most frequent, but potential serious manifestations were also present. These manifestations were similarly distributed between patients with Norwegian and non-Norwegian ancestry (Figure 1).References[1]Davatchi F. et al. Behcet`s disease: epidemiology, clinical manifestations and diagnosis. Expert Review of Clinical Immunology, 13:1, 57-65, DOI: 10.1080/1744666X.2016.1205486Disclosure of InterestsNone declared
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